Saturday, January 31, 2015

"Our deepest fear is not that we are inadequate. Our deepest fear is that we are powerful beyond measure. It is our light, not our darkness that most frightens us. We ask ourselves who am I to be brilliant, gorgeous, talented, fabulous? Actually, who are you not to be? Your playing small does not serve the world. There is nothing enlightened about you shrinking so that other people won't feel insecure around you. We are all meant to shine like children do. We were born to make manifest the glory that is within us. It's not just in some of us, it's in everyone. And as we let our own light shine we unconsciously give other people permission to do the same. As we are liberated from our own fear, our presence automatically liberates others."

Almost 5 years ago, on March 14, 2010, our lives forever changed as we welcomed two beautiful babies into our family. We found out just a few days later that Drew had cystic fibrosis, a life shortening genetic disease that does not have a cure. I can't even describe the overwhelming sense of fear that accompanies that diagnosis.

I remember so vividly jumping into fundraising as we were told that our dollars went right to research to cure this disease, and that the Cystic Fibrosis Foundation was our only hope. We walked in our first Great Strides just days after Drew was released from the NICU.

He came home with an ileostomy and a list of instructions for how to handle his bowls that were protruding from his abdomen. We macgyver our way through daily ostomy bag changes, becoming better at them than the seasoned nurses who provided regular home care. We were given a pricey special pre-digested formula and a print-out of instructions for how to mix it so that it would be higher calorie and meet his nutritional needs. We were given a nebulizer and other equipment to use with the countless new medications that were prescribed. We had a photocopy of a diagram describing how we should beat on our sons back to dislodge the mucus that was already starting to clog his airways. Our tupperware cabinet quickly turned into our medicine cabinet and our home looked like an assisted living facility. We were measuring stool with a syringe and calculating enzyme dosages on our iPhones. We scrambled as we learned what we needed to do and when we needed to do it. We were completely and totally exhausted, and we were never given time to grieve the loss of the life we had expected. We were not trained for this. Elizabeth Scarboro described it so perfectly in her book "My Foreign Cities: A Memoir" when she said of caregiving, "My field was large and nebulous - I had no idea how to do most of the things in it, and all I could do was try anyway." And try we did.

As time passed, things got easier. Not in the sense of there being less to do, it simply became more of a routine. We continued to be involved with the Cystic Fibrosis Foundation and I found a new role as the State Advocacy Chair. Initially intimidated by the congresspeople that I would meet with, I quickly realized that they are people just like you and I. I was never politically saavy, but I didn't have to be. When I would meet with them, I thought less about what I wanted them to know and more about how I wanted them to feel when I left our meetings. I wanted the world to be on Team Drew, and I found that sharing our story in all its simple yet oh so complicated glory was helping me and it was helping others. They now had a person that they were thinking about when they were voting on things like whether to support the creation of the National Center for Advancing Translation Sciences, a branch of the National Institute of Health that would accelerate drug development for people like Drew who did not have time to wait.

The more that I worked at this role, the more I learned. I joined the advisory council at our hospital and I refused to settle for the status quo. I wouldn't accept what I believed was anything less than the full potential of that organization. I had to try, to keep trying, for Drew and for the other patients and families who didn't have the time or the voice or the courage to push these issues like I did. I was very fortunate to be among such amazing people Cincinnati Children's Hospital who were receptive to new people and new ideas, and embraced me as a partner on their team. I served, and continue to serve, as a constant reminder of why they are doing the work that they do and I implored, let patient's help. Team Drew was growing.

I had a vision of a better way and the more I shared my vision, the more people wanted to hear about it. It seemed such an aspirational goal 3 years ago, to have patients working alongside clinicians and researchers, combining the knowledge they had with the molecular biology and genetics expertise of some of the top researchers in the world. I had data, in my head and in my heart, that helped me to manage Drew's health, and I believed that we could get farther together than any of us could get alone. Then in 2012, at the first North American Cystic Fibrosis Conference that I the opportunity to attend, I heard something that would catapult me forward. The scientists and researchers working so tirelessly to cure this disease felt that the missing puzzle piece was understanding what happened to patients between clinic visits. I thought to myself "I know that! I have that data! I live that every day!". I realized that the cure for cystic fibrosis would come through partnership. The efforts that are made on behalf of patients and families cannot go unappreciated, but alone they are not enough. We need to work together through every stage and every step of the way.

I've had the great fortune of speaking everywhere from San Francisco to Switzerland and many places in between, advocating for patients and families to be included as partners in everything from clinical trial design to helping clinicians accept the idea that some people with CF may prioritize quality of live over longevity. While the cognitive complexity of adding another medication or therapy to our daily regimen may be small, the burden becomes too much to bear. There is incredible value in a humble awareness of a shared humanity. We are all just people striving to do and be well and I view my role as one to ensure that that is realized and appreciated.

On Tuesday evening, January 27th, I received the following email:

INVITATION: White House Event

The Office of Science and Technology Policy is pleased to invite you to an event at The White House in the morning of Friday, January 30.

Details
DATE: Friday, January 30, 2015
TIME: Morning TBD
LOCATION: The White HouseAdditional logistics will be made available upon receipt of your RSVP

That was the email in its entirety. I wanted to go, but I had no idea what this was about. Logistically it would be a tough thing to coordinate in 2 days time. I had just earlier that week sat down to define my mission path, to create some structure for all of the different work that I was doing and help to ensure that the opportunities I would choose to pursue going forward would be ones that fed my soul. I had no idea what this "White House Event" was about, but we decided that I should probably go and find out.

I used sky miles to book a ticket and invited myself to stay with a friend who lives in town. I would leave here on Thursday night and return on Friday afternoon. That same day, two kids got sick, our only 6 person vehicle broke, and the exhaustion that is a Wednesday night in our world started to set in. I said out loud that I should have just said no. There is always another opportunity and if I didn't make it to this one I was confident that another would follow. I was mad at myself for being "wooed" by the subject line of an email from a sender I didn't even know to an event that I knew nothing about. We got the kids to bed and then everything changed.

At 8pm on Wednesday, January 28th, I received an email that changed everything. "We are pleased to confirm your attendance at an event On the morning of Friday, January 30 with the President highlighting investments to improve health and treat disease." You guys, THE FREAKING PRESIDENT! Of The United States of America!! My advocacy efforts, in just 5 short years, had taken me from being a scared parent to being invited to attend what I hope will become known as historic event for rare and orphan diseases at the White House with the President of the United Freaking States. !!!!!!.

The very first thought that I had (and no I'm not kidding) was OMG I have nothing to wear! I hopped in the car and hightailed it to Macys, the closest store to our house, and the only one still open at 9:00 at night. I had less than 24hrs to pull this all together before I would board a plane to Washington, DC, and in our world that 24hrs includes multiple school drop offs and pick ups, swim lessons and gymnastics, story reading and lego building and working, and lets not forget those 3 hours of breathing treatments and airway clearance. I FaceTimed with a dear friend who helped me to pick out the perfect outfit via a virtual fashion show, I packed my bags, and I left for DC.

Tuesday, January 20, 2015

For many, many months now (as in enough months to make up years), I've been talking about how we can improve the current system for cystic fibrosis care. I've been testing and trying new things on my own, and dreaming about what CF life and care could and should look like. Here's a collection of blog posts with my thoughts on the topic (in chronological order) that also help to put into context some of what I'm about to tell you about:

This is actually happening! Last week, I spent 2 days in Bethesda with 40 other folks - CF parents, patients, clinicians, researchers, industry innovators - all with the same desire: to improve both life and care in the CF community.

The first step in creating this new system is to deconstruct the current system to better understand the components - whats currently working in CF life and care and what's not. We got (and continue to get) this information from the community - from blogs and Facebook groups and testimonials from the patients and families in the room. Then with all of this knowledge, we imagined what "the perfect system" would look like and collectively came up with our dream list which included things like:

100% of patients and families feel that there is research happening relevant to their needs

Everyone has access to own personal data and a personalized treatment plan that is accessible to whomever they choose to share it with

There is mutual trust, collaboration and empathy where all patients/ families/ professionals think that their skills and knowledge have contributed to the decisions and actions that matter to them

No more waiting rooms

Doing your treatments should be like brushing your
teeth. It’s a habit and there is no hassle.It’s convenient and portable.

Over the next couple of months we will figure out if some of the targets we laid out are the right ones that can lead us to better life balance, health management and care coordination by finding or creating different solutions and tools to see how they might help to achieve some of our "perfect system" goals. There are many good parts about the current model of CF care that either aren't known about or aren't implemented properly, so we are going out into the community to learn more about what works, what doesn't, and what we need to do to create the CF Care Model of the Future.

My goal of 2015 is to blog more, share more of this work that I find so amazing, and connect with people whose perspectives need to be heard. Twitter is an amazing vehicle for sharing, and the project is using the hashtag #CFBigIdea. Join us!! Another platform that we are using (which I will talk about in more detail in another post) is called Smart Patients. The recent creation of Learn from Smart Patients creates a feedback look where clinicians can learn from the conversations that patients are having with one another, in a safe and privacy respecting way. The real value in this platform versus something like Facebook is that the conversations aren't happening inside of an echo chamber; we're starting to talk with the people that can collaborate with us to help us overcome the barriers we face [that they don't know we are facing if we only talk about them within a closed group]. Don't get me wrong, there is tremendous value in the empathy and support that comes from a group like CF Mamas. I in no way want any of this to replace that.

I want to talk to you, fellow CF mama warriors and amazing patients and patient advocates. The best way to reach me for questions/comments/concerns is through Twitter, I'm @ekeeleymoore (I'm into learning out loud so that others can reap the benefit - chances are someone else is wondering the same thing you are, and we can all learn something through having that conversation together) or via email at erin(dot)moore(at)cchmc(dot)org.

It's been so long since I've blogged that I don't even know where to start! I guess I'll tell you a little bit about what's been going on with Drew, and then either after that or in a separate post share a little bit about some of the work that I've been doing lately.

I think I left off with the PICC that Drew had for IV antibiotics in November (seriously, how is it already January!). While the antibiotics didn't seem to make much of a noticable difference in his cough, the cycling of inhaled Ceftazadime and Tobi constantly since the PICC was removed has seemed to help. We are now at 3 hours of treatments a day. Every. Single. Day. Sitting through all of that with little to no complaining is Drew's superpower.

We saw the doctor in January for a clinic visit, and while our cough was nearly non-existant, nasal congestion was at a epic high since his sinus surgery when he was two. The doctor said that he was full of nasal polyps and it was likely time for another surgery. She referred us to his ENT for another opinion and likely scheduling of the procedure (less than pleasant I tell you).

At the NACFC this fall, Drews doctor and I had also talked a little bit about HGH (human growth hormone). Kids with CF often tend to have lower than average levels of this hormone and sometimes need a supplement. The supplementation is often useful in increasing appetite and weight gain, and during the conference we also discussed how it may be useful in promoting lung growth, something that we want and need. Drew has always had low level of HGH, but his growth is mostly on track, with BMI falling in the 50th percentile or above since he was about 6mo old (I still have the growth chart from the dietician from when he hit the 50th!). Well, a few months ago with his annual labs we found once again that his HGH is low. Prior to our visit, his doctor had consulted with his endocrinologist about whether HGH supplementation was necessary. This comes in the form of a shot (I'm not sure if its daily or weekly?) but I was dreading having something else to manage, even if it could help him. The even bigger headache, I feared, was the rumors that I'd heard about the difficulty of getting this approved by insurance. I've fought the good fight, and i'll do it again if I have to.

To my surprise, the endocrinologst almost laughed at the notion of putting him on HGH given his current growth curve. However, when our doctor shared some of what she had learned at the NACFC about it helping to promote lung growth, we all decided that it would be best to have a follow up appointment and look at all of the different things that go into making this decision. That appointment is coming up in March.

After the appointment, I started to think about Drew's sinus congestion. We hadn't been doing the nasal sprays as often as we should, but quite frankly given the new 3hr treatment times that we make him sit through, we have decided to spare him the nasal cleansing, which his absolutely hates. It sounds silly, but almost worth an awful one day surgery and a long span of no issues to forgo 30 seconds of screaming and squirming and spraying a series of medicines up his nose.

Then I had an "ah-ha!" moment. After Drew's last surgery, his doctor suggested that without regular cleanses he would likely need this surgery every 6-12mo. In thinking of alternatives, I signed him up for swim lessons. Drew has been swimming, at least weekly, since his last surgery, and then in November when he got his PICC and was out of the pool for 8-10 weeks, all of this sinus stuff returned. Well, I got that kid back in the pool faster than I've ever done anything in my life, and wouldn't you know, the sinus issues disappeared!

Because we had already scheduled the ENT appointment, we went, just this past Saturday, and the doctor said that he has no polyps and there is no reason or need for surgery. I told him about the pool, and he [who has poor bedside manner] not so politely stated that there is no scientific evidence that chlorine can shrink polyps. Now I'm no doctor, but 3 weeks prior there was a nose full of polyps, so many in fact that we were getting ourselves ready to schedule a surgery. We had tried some sprays and antibiotic drops that didn't so much, and it was not until this kid was in the pool that his nose cleared right up. I'm not suggesting that this will work for everyone, but I also don't want to dismiss the fact that it worked for us. I bet my YMCA membership would cost my insurance company less than a sinus surgery. Just sayin'.

Overall, Drew is doing really well. I would like to get off some of these inhaled antibiotics and get some of my time back, but right now it's working, so for the foreseeable future we shall keep on keepin' on. With registration right around the corner, the thought of Kindergarten lurking in the back of my mind makes me nauseous (thoughts for another post).

Saturday, January 17, 2015

The blog is still a thing. Everyone in the house has a stomach bug. I have a longer story about how we're avoiding another sinus surgery, but the punchline is that we're avoiding another sinus surgery. My resolution was to blog more, even if it has to be just a few sentence update, I want to blog more. That's all i've time for tonight. Stay tuned!

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org